TOPICS: Maple syrup urine disease, autosomal recessive disorder, branched-chain amino acids, isoleucine, leucine, valine, branched-chain alpha-ketoacid dehydrogenase complex (BCKDC), alpha-keto acid, acetyl CoA, propionyl CoA, methylmalonyl CoA, cofactors, thiamine (vitamin B1), lipoic acid, coenzyme A (vitamin B5), FAD (vitamin B2), NAD (vitamin B3), neurological symptoms, phenylketonuria, phenylalanine hydroxylase deficiency, dihydrobiopterin reductase, neurotransmitters, seizures, intellectual disability, musty odor, catecholamine synthesis, phenylalanine, DOPA, dopamine, DOPA decarboxylase, Carbidopa, Parkinson’s disease, norepinephrine, epinephrine, succinyl CoA, methylmalonyl-CoA mutase, phenylethanolamin-N-methyltransferase (PNMT), cortisol, albinism, tyrosinase, melanin, melanocyte, skin cancer, alkaptonuria, homogentisic acid dioxygenase deficiency, homogentisic acid, pigmented deposition, arthritis, black urine, tetrahydrobiopterin (BH4), dihydropbiopterin (BH2), dihydrobiopterin reductase
